title: Severe persistent bone marrow failure following therapy with 2-chlorodeoxyadenosine for relapsing juvenile xanthogranuloma of the brain.
abstract: 2-Chlorodeoxyadenosine (2-CdA) has been successfully used in children to treat refractory Langerhans cell histiocytosis and juvenile xanthogranuloma (JXG) as salvage therapy. Although 2-CdA is generally well-tolerated, with temporary myelosuppression as the primary dose-limiting toxicity, prolonged myelosuppressive, and immunosuppressive effects have been reported. We describe an adolescent patient with refractory multiple central nervous system JXG, with the lesion size markedly reduced after treatment with 2-CdA. However, severe transfusion-dependent bone marrow failure developed after five courses of 2-CdA. He underwent successful bone marrow transplantation from his HLA compatible sister with reduced intensity conditioning.
authors.affiliation: Department of Hematology Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan. kayoyamada@msn.com
authors.forename: Kayo
authors.initials: K
authors.lastname: Yamada
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yasui
authors.forename: Akihisa
authors.initials: A
authors.lastname: Sawada
authors.forename: Masami
authors.initials: M
authors.lastname: Inoue
authors.forename: Masahiro
authors.initials: M
authors.lastname: Nakayama
authors.forename: Keisei
authors.initials: K
authors.lastname: Kawa
mesh_terms: D000741:Anemia, Aplastic; D001855:Bone Marrow Diseases; D000080983:Bone Marrow Failure Disorders; D016026:Bone Marrow Transplantation; D001927:Brain Diseases / Q000150:complications* / Q000628:therapy; D002648:Child; D017338:Cladribine / Q000009:adverse effects*; D006457:Hemoglobinuria, Paroxysmal / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects*; D008297:Male; D011379:Prognosis; D012008:Recurrence; D065227:Transfusion Reaction; D014972:Xanthogranuloma, Juvenile / Q000150:complications* / Q000628:therapy
chemical_list: D007166:Immunosuppressive Agents; D017338:Cladribine